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Dr James Galvin

Special reports

Author: Geoffrey ChangPublished: 19 November 2015

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Amid revelations that Robin Williams also suffered from Lewy body dementia, as well as Parkinson’s disease, before his death last year, we shed light on the little-known dementia, often misdiagnosed as Parkinson’s and described by experts as “the most common disease you have never heard of”

“Lewy body dementia killed Robin – it’s what took his life,” said Susan Schneider, the wife of Robin Williams, speaking publicly for the first time since her husband’s death.

Not heard of Lewy body dementia or LBD before? You’re not alone. According to Dr James Galvin, a neurology and psychiatry professor at Florida Atlantic University: “It’s the most common disease you have never heard of.”

Sometimes referred to as ‘Parkinson’s with Lewy bodies’ or ‘Dementia with Lewy bodies’, it’s the second-most common dementia after Alzheimer’s, affecting memory, movement and motor control, while also causing visual hallucinations and severe psychiatric symptoms.

Disguised dementia

It’s a disease described by Jacqueline Cannon of the Lewy Body Society as “the worst bits of Alzheimer’s and the worst bits of Parkinson’s put together”. The symptomatic similarities that LBD shares with these diseases means it is often initially misdiagnosed as one or the other. It is, however, considered far more brutal and viciously progressive.

While the patient is living, the diagnosis process is not conclusive, with tests that are only able to rule out other conditions by deduction. Absolute diagnosis can only be confirmed after death if Lewy bodies are found in the brain at post-mortem examination.

Discovering LBD

While researching Parkinson’s disease in 1912, German neurologist Freiderich H Lewy (who was a colleague of Dr Alzheimer) discovered small deposits of the alpha-synuclein protein present in the brain cells of people with the condition. These proteins were later named Lewy bodies and are occur in both LBD and Parkinson’s.

The deposits develop inside some nerve cells (neurons) in the brain at the synapses, interrupting messaging and causing neurons to die. The patient could develop dementia if they are found in the cortex, or Parkinsonism if found in the brain stem.

“A sea monster with 50 tentacles of symptoms”

“This disease is a sea monster with 50 tentacles of symptoms that show when they want,” Schneider said of LBD. “We were living a nightmare.”

The long list of unpredictable symptoms includes: impaired visual perception and spatial awareness, delusions, hallucinations and loss of motor control.

One of the main differences to Alzheimer’s disease is that the patient is aware of the mental deterioration, that they are losing their mind. And the more aware they are of the situation, the more likely depression is to worsen.

“Robin was very aware that he was losing his mind and there was nothing he could do about it”

Schneider added: “Robin was very aware that he was losing his mind and there was nothing he could do about it.”

Jacqueline Cannon said of her father’s condition: “He always used to say to me, ‘I’m losing my mind’. We say to people that LBD is not just about memory. It’s about the other symptoms that go with it, especially the hallucinations.”

Professor Ian McKeith, president of the Lewy Body Society, believes there is cause for hope however. In a piece published by The Conversation, he wrote: “Therapeutic trials have been few and far between in LBD because of a combination of a lack of compounds to test, a pre-occupation with targeting Alzheimer’s and a reluctance of regulatory bodies to recognise LBD. All of these are now changing and LBD is increasingly viewed as a malleable and commercially-viable target.”

IN THE NEWS

Carefully selected news stories from the international Parkinson's community.

3 weeks ago

Excess calcium in brain could cause Parkinson’s

Researchers at the University of Cambridge, UK, have discovered that excess levels of calcium in brain cells may lead to the formation of the toxic clusters that signify Parkinson’s disease. The findings, reported in the journal ‘Nature Communications’, show that calcium can influence the interaction between small membranous structures inside nerve endings, which are important for neuronal signaling in the brain, and alpha-synuclein – the protein associated with Parkinson’s disease. Dr Janin Lautenschläger, the paper’s first author, said: “This is the first time we’ve seen that calcium influences the way alpha-synuclein interacts with synaptic vesicles. We think that alpha-synuclein is almost like a calcium sensor. In the presence of calcium, it changes its structure and how it interacts with its environment, which is likely very important for its normal function.”

Jewish people with Crohn’s disease more likely to carry LRRK2 gene mutation

A scientific study has concluded that there may be a link between Parkinson’s and Crohn’s disease within the Ashkenazi Jewish community. The study’s findings, which were published in the journal ‘Science Translational Medicine’, has found that members of the population with Crohn’s disease are more likely to carry the LRRK2 mutation which is a significant cause of Parkinson’s. Lead researcher Dr Inga Peter, professor of genetics and genomic sciences at the Icahn School of Medicine, New York, US, said: “Crohn’s disease is a complex disorder with multiple genes and environmental factors involved, which disproportionately affects individuals of Ashkenazi Jewish ancestry. “The presence of shared LRRK2 mutations in patients with Crohn’s disease and Parkinson’s disease provides refined insight into disease mechanisms and may have major implications for the treatment of these two seemingly unrelated diseases.”

Could caffeine in the blood help diagnose Parkinson’s?

Blood caffeine levels could be promising diagnostic biomarkers for early-stage Parkinson’s, Japanese researchers reported in the journal ‘Neurology’ earlier this month. The study found that people with Parkinson’s had lower levels of caffeine and caffeine metabolites in their blood than people without the disease, at the same consumption rate. Caffeine concentrations also were decreased in Parkinson’s patients with motor fluctuations than in those without Parkinson’s. However, patients in more severe disease stages did not have lower caffeine levels. The study’s authors, Dr David Munoz, University of Toronto, and Dr Shinsuke Fujioka, Fukuoka University, suggested that the “decrease in caffeine metabolites occurs from the earliest stages of Parkinson’s.” They added: “If a future study were to demonstrate similar decreases in caffeine in untreated patients with Parkinson’s […] the implications of the current study would take enormous importance.”